Complement activation and renal dysfunction in patients with acquired thrombotic thrombocytopenic purpura.

Letter to Blood| May 4, 2023 Complement activation and renal dysfunction in patients with acquired thrombotic thrombocytopenic purpura Clinical Trials & Observations Massimo Cugno, Massimo Cugno 1Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy2Department of Internal Medicine, UOC Medicina Generale–Emostasi e Trombosi, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy https://orcid.org/0000-0002-9918-0763 Search for other works by this author on: This Site PubMed Google Scholar Ilaria Mancini, Ilaria Mancini 1Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy2Department of Internal Medicine, UOC Medicina Generale–Emostasi e Trombosi, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy Search for other works by this author on: This Site PubMed Google Scholar Dario Consonni, Dario Consonni 3Epidemiology Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy https://orcid.org/0000-0002-8935-3843 Search for other works by this author on: This Site PubMed Google Scholar Valentina De Zan, Valentina De Zan 1Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy Search for other works by this author on: This Site PubMed Google Scholar Gianluigi Ardissino, Gianluigi Ardissino 4Center for HUS Prevention, Control and Management at Pediatric Nephrology, Dialysis and Transplant Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy Search for other works by this author on: This Site PubMed Google Scholar Samantha Griffini, Samantha Griffini 2Department of Internal Medicine, UOC Medicina Generale–Emostasi e Trombosi, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy Search for other works by this author on: This Site PubMed Google Scholar Elena Grovetti, Elena Grovetti 2Department of Internal Medicine, UOC Medicina Generale–Emostasi e Trombosi, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy Search for other works by this author on: This Site PubMed Google Scholar Luigi Porcaro, Luigi Porcaro 5Medical Genetics Laboratory, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy https://orcid.org/0000-0003-3172-6528 Search for other works by this author on: This Site PubMed Google Scholar Barbara Ferrari, Barbara Ferrari 2Department of Internal Medicine, UOC Medicina Generale–Emostasi e Trombosi, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy Search for other works by this author on: This Site PubMed Google Scholar Andrea Artoni, Andrea Artoni 2Department of Internal Medicine, UOC Medicina Generale–Emostasi e Trombosi, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy Search for other works by this author on: This Site PubMed Google Scholar Flora Peyvandi Flora Peyvandi 1Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy2Department of Internal Medicine, UOC Medicina Generale–Emostasi e Trombosi, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy https://orcid.org/0000-0001-7423-9864 Search for other works by this author on: This Site PubMed Google Scholar Blood (2023) 141 (18): 2278–2282. https://doi.org/10.1182/blood.2022018644 Article history Submitted: October 6, 2022 Accepted: February 13, 2023 First Edition: March 6, 2023 Share Icon Share Facebook Twitter LinkedIn Email Tools Icon Tools Request Permissions Cite Icon Cite Search Site Citation Massimo Cugno, Ilaria Mancini, Dario Consonni, Valentina De Zan, Gianluigi Ardissino, Samantha Griffini, Elena Grovetti, Luigi Porcaro, Barbara Ferrari, Andrea Artoni, Flora Peyvandi; Complement activation and renal dysfunction in patients with acquired thrombotic thrombocytopenic purpura. Blood 2023; 141 (18): 2278–2282. doi: https://doi.org/10.1182/blood.2022018644 Download citation file: Ris (Zotero) Reference Manager EasyBib Bookends Mendeley Papers EndNote RefWorks BibTex toolbar search Search Dropdown Menu toolbar search search input Search input auto suggest filter your search All ContentAll JournalsBlood Search Subjects: Clinical Trials and Observations, Thrombosis and Hemostasis, Vascular Biology TO THE EDITOR: Thrombotic thrombocytopenic purpura (TTP) is a rare life-threatening thrombotic microangiopathy characterized by nonimmune hemolytic anemia, thrombocytopenia, and central nervous system abnormalities1,2; however, multiorgan involvement and renal dysfunction1-4 may occur. The disease is due to the deficiency of a plasma metalloprotease named ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) that cleaves ultralarge von Willebrand factor (VWF) multimers.2 In TTP, the excess of ultralarge multimers of VWF, which are not cleaved anymore by ADAMTS13, substains a thrombotic condition via endothelial injury and intraluminal platelets thrombosis, causing platelet consumption, organ damages, and mechanical hemolysis.5 The defect of ADAMTS13, in the majority of cases, is acquired because of the development of anti-ADAMTS13 autoantibodies (immune-mediated TTP [iTTP])6 or, in very few cases, may be genetically determined as autosomal recessive... References 1.Moake JL. Thrombotic microangiopathies. N Engl J Med. 2002;347(8):589-600.Google ScholarCrossrefSearch ADS 2.Kremer Hovinga JA, Coppo P, Lämmle B, Moake JL, Miyata T, Vanhoorelbeke K. Thrombotic thrombocytopenic purpura. 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